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Biomedical subjects

Camille Francès

Publications and source records attributed to Camille Francès.

15 recordsLinked to original sources
PubMed2006

Low blood concentration of hydroxychloroquine is a marker for and predictor of disease exacerbations in patients with systemic lupus erythematosus.

OBJECTIVE: To study the possible relationship between whole-blood hydroxychloroquine (HCQ) concentrations and clinical efficacy of HCQ in patients with systemic lupus erythematosus (SLE). METHODS: Whole-blood HCQ concentrations were measured, under blinded conditions, in 143 unselected patients with SLE who had been receiving HCQ 400 mg daily for at least 6 months. The relationship of these concentrations to current disease activity and to subsequent exacerbations during 6 months of followup was investigated. RESULTS: At baseline, 23 patients had active disease (mean +/- SD SLE Disease Activity Index 12.4 +/- 7.5). The mean whole-blood HCQ concentration in this group was significantly lower than that in the 120 patients with inactive disease (694 +/- 448 ng/ml versus 1,079 +/- 526 ng/ml; P = 0.001). Among the 120 patients who had inactive disease at baseline, the mean HCQ concentration at baseline in the 14 (12%) who had disease exacerbations during followup was significantly lower than that in the patients whose disease remained inactive. Multivariate logistic regression showed that the HCQ concentration was the only predictor of exacerbation (odds ratio 0.4 [95% confidence interval 0.18-0.85], P = 0.01). Receiver operating characteristic curve analysis showed that a whole-blood HCQ concentration cutoff of 1,000 ng/ml had a negative predictive value of 96% for exacerbation during followup. CONCLUSION: Low whole-blood HCQ concentrations are associated with SLE disease activity and are a strong predictor of disease exacerbation. Regular drug assaying and individual tailoring of treatment might help to improve the efficacy of HCQ treatment in patients with SLE.

Adult↗
PubMed2005

Dermatologic manifestations of the antiphospholipid syndrome: two hundred consecutive cases.

OBJECTIVE: To describe dermatologic manifestations of the antiphospholipid syndrome (APS) and to investigate possible correlations between livedo reticularis and other APS manifestations. METHODS: We conducted a single-center study of 200 consecutive patients with primary or systemic lupus erythematosus-related APS. To qualify for the study, patients had to fulfill clinical and laboratory criteria from the most recent international consensus statement on classification of definite APS. Dermatologic manifestations were systematically evaluated by a dermatologist. Only dermatologic lesions that may be related to APS were included in the analyses. Correlations between livedo reticularis and other APS manifestations were determined using Fisher's 2-tailed, chi-square, and nonparametric Mann-Whitney tests. RESULTS: Dermatologic manifestations were noted in 49% of the patients and were the presenting manifestations in 30.5%. Livedo reticularis was the most frequent manifestation, observed in 25.5% of the patients. Livedo reticularis was shown to be significantly associated with cerebral or ocular ischemic arterial events (odds ratio [OR] 10.8, 95% confidence interval [95% CI] 5.2-22.5), seizures (OR 6.5, 95% CI 2.6-16), all arterial events (OR 6, 95% CI 2.9-12.6), heart valve abnormalities detected on echocardiography (OR 7.3, 95% CI 3.6-14.7), and arterial systemic hypertension (>/=160/90 mm Hg) (OR 2.9, 95% CI 1.5-5.7). Conversely, it was observed with decreased frequency in patients with only venous thrombosis (OR 0.2, 95% CI 0.1-0.5). CONCLUSION: The dermatologic manifestations of APS are frequently the presenting feature of the syndrome, and livedo reticularis is significantly associated with the arterial subset of APS.

Adolescent↗
PubMed2005

Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study.

OBJECTIVE: Screening allows for early management of pulmonary arterial hypertension (PAH), a severe complication of systemic sclerosis (SSc). Since no consensus has been reached on the method and criteria for optimal screening, we sought to develop an algorithm based on symptoms, Doppler echocardiography, and right heart catheterization (RHC) for application to a nationwide multicenter SSc population in France. METHODS: This prospective study was conducted from September 2002 to July 2003 by experts at 21 SSc centers. At each center, SSc patients without severe pulmonary function abnormalities underwent Doppler echocardiography by an experienced cardiologist. Patients with a peak velocity of tricuspid regurgitation (VTR) of >3 meters/second or 2.5-3 meters/second with unexplained dyspnea were asked to undergo RHC to confirm PAH according to international guidelines. RESULTS: Of the 599 patients analyzed, 29 had known PAH and 33 had suspected PAH, based on Doppler echocardiography, and underwent RHC. Of these 33, 18 were found to have PAH, 3 had left ventricular dysfunction, and 12 had no PAH. Newly diagnosed cases of PAH were of mild severity (mean +/- SD pulmonary artery pressure [mPAP] 30 +/- 9 mm Hg, mean +/- SD total pulmonary resistance [TPR] 524 +/- 382 dynes x second/cm(5)). Hemodynamic findings in patients with known PAH were mPAP 49 +/- 17 mm Hg and TPR 1,007 +/- 615 dynes x second/cm(5). The estimate of PAH prevalence was 7.85% (95% confidence interval 5.70-10.00). CONCLUSION: This screening algorithm, based on dyspnea, Doppler echocardiographic evaluation of VTR, and RHC, enabled early detection of PAH at a mild stage. Whether mild PAH will evolve to severe PAH in reported cases and whether this early diagnosis translates into improved prognosis for patients with mild PAH will be evaluated in the ongoing 3-year followup of this cohort.

Adult↗
PubMed2005

Skin metastases from unknown origin: role of immunohistochemistry in the evaluation of cutaneous metastases of carcinoma of unknown origin.

Determining the primary origin of skin metastases might be a challenging issue for pathologists, especially when there is no primary history or when this history is unavailable. The poor specificity of morphological appreciation is challenging, emphasizing the need for ancillary studies. We have retrieved 44 cases of skin metastases from our pathology files. Paraffin blocks were collected and homemade tissue arrays were made. We have tried to assess the primary origin based on morphological data alone, and then using 13 antibodies (cytokeratins (CK) 5/6, 7, 19, 20, thyroid transcription factor-1, carcinoembryonic antigen, PS100, tumor-associated glycoprotein 72, BerEP4, estrogen receptor (ER), progesterone receptor (PR), CD10, and E-cadherin). Most metastases in our series were from breast (13) and colorectal cancers (six) as they are the main clinical activity in our hospital. Only 44% of cases were correctly assessed based on the sole morphology, emphasizing the need for ancillary studies. CK 20, ER, and PR were the most helpful markers to determine the primary origin of skin metastases by highlighting colorectal origin and mammary origin, respectively. By far, clinical information and morphological evaluation are more reliable than the use of ancillary techniques, which have to be used in the absence of the former one and the poor differentiation of the latter ones. Azoulay S, Adem C, Le Pelletier F, Barete S, Francès C, Capron F. Skin metastases from unknown origin: role of immunohistochemistry in the evaluation of cutaneous metastases of carcinoma of unknown origin.

Adenocarcinoma↗
PubMed2005

Tumor antigen markers for the detection of solid cancers in inflammatory myopathies.

Dermatomyositis and polymyositis patients have an increased risk of developing cancers. We have assessed the diagnostic values of serum tumor markers for the detection of solid cancer in dermatomyositis/polymyositis patients. Serum carcinoembryonic antigen, CA15-3, CA19-9, and CA125 were assayed by immunoradiometric methods in 102 dermatomyositis/polymyositis patients. All the patients had complete physical examination, chest X-ray, echocardiogram, gastrointestinal tract endoscopic explorations, thoracoabdomino-pelvic computed tomography scan, and all women had gynecologic examination and mammogram. Exclusion criteria for study were childhood dermatomyositis, inclusion body myositis, myositis associated with a connective tissue disease, prior history of cancer, and the presence of benign conditions known to elevate serum tumor markers. After a median follow-up of 59 months, 10 (9.8%) patients had a solid cancer. Initial elevation of CA125 was associated with an increased risk of developing solid cancer [P = 0.0001 by Fisher's exact test; odds ratio (OR), 29.7; 95% confidence interval (95% CI), 8.2-106.6]. For CA19-9, there was a trend towards a significant association (P = 00.7; OR, 4.5; 95% CI, 1-18.7, respectively). Diagnostic values of elevated CA125 and CA19-9 at screening increased when the study analysis was restricted to patients who developed a cancer within 1 year (P < 0.0001 and P = 0.018, respectively) or to patients without interstitial lung disease (P = 0.00001; OR, 133; 95% CI, 6.5-2733 and P = 0.027; OR, 9; 95% CI, 1.5-53, respectively). Individual comparisons of the baseline and the second CA125 value showed that three of the eight patients with cancers versus 3 of the 76 patients without, displayed an increase of their CA125 level (P = 0.01 by Fisher's exact test). We conclude that CA125 and CA19-9 assessment could be useful markers of the risk of developing tumors for patients with dermatomyositis and polymyositis and should therefore be included in the search for cancer in dermatomyositis/polymyositis patients, especially for patients without interstitial lung disease.

Adult↗
PubMed2004

Protein Z deficiency in antiphospholipid-negative Sneddon's syndrome.

BACKGROUND: Sneddon's syndrome is characterized by the association of ischemic cerebrovascular events and widespread livedo racemosa. The pathophysiology of Sneddon's syndrome remains elusive, but various prothrombotic abnormalities have been previously reported in this setting. Low levels of protein Z, a downregulator of coagulation, have been recently linked to an increased risk of arterial thrombosis. The purpose of this study was to investigate the levels of protein Z in a series of Sneddon's syndrome patients without circulating antiphospholipid antibodies in comparison with an age- and sex-matched control population. METHODS: Twenty-six patients and 78 healthy controls had determination of their protein Z blood levels by an enzyme-linked immunoassay test. Patients' thrombotic and vascular risk factors, including tobacco smoking, arterial hypertension, oral contraceptive agents, dyslipidemia, factor V Leiden, and factor II mutation were recorded. RESULTS: Protein Z plasma levels were significantly lower in patients (mean 1.47 mg/L) than in controls (mean 1.93 mg/L) (P=0.02). Prevalence of protein Z deficiency (level <1 mg/L) was significantly higher (P=0.001) among patients (31%) than among controls (3.8%). Factor V Leiden and heavy smoking were observed in 4 and 7 patients, respectively. CONCLUSIONS: Sneddon's syndrome could be viewed as the peculiar clinical expression of various and sometimes associated coagulation abnormalities. Low levels of protein Z may account, at least partly, for the thrombotic events observed in Sneddon's syndrome and shed a new light on its pathophysiology. Clinical implications for protein Z deficiency in this setting deserve further investigations.

Adult↗
PubMed2004

Antineutrophil cytoplasmic antibodies of IgA class in neutrophilic dermatoses with emphasis on erythema elevatum diutinum.

OBJECTIVE: To evaluate the prevalence of IgA and IgG antineutrophil cytoplasmic antibodies (ANCAs) in erythema elevatum diutinum in comparison with 2 other groups of neutrophilic dermatoses: Sweet syndrome and pyoderma gangrenosum. DESIGN: Detection of IgA and IgG ANCAs in the serum of patients with neutrophilic dermatoses and characterization of the previously known antigenic targets. SETTING: All serum was analyzed without knowledge of diagnosis in the Immunology Department, Pitié-Salpêtrière Hospital, Paris, France. PATIENTS: Ten patients with erythema elevatum diutinum, 10 with Sweet syndrome, 10 with pyoderma gangrenosum, and 10 healthy volunteers. MAIN OUTCOME MEASURES: IgA and IgG ANCAs were sought by indirect immunofluorescence with ethanol and formaldehyde-fixed human neutrophil preparations as the substrate. Enzyme-linked immunosorbent assays were further performed for antigen characterization. RESULTS: IgA ANCAs were observed in 60% and IgG ANCAs in 10 (33%) of the patients. All patients with erythema elevatum diutinum had IgA ANCAs. IgA fluorescence in formaldehyde-fixed neutrophils was restricted to those from patients with erythema elevatum diutinum. Enzyme-linked immunosorbent assays disclosed no single predominant target, and antigens remained largely undetermined in erythema elevatum diutinum. CONCLUSIONS: The ANCAs, particularly of IgA class, may prove to be a helpful paraclinical marker in erythema elevatum diutinum and an interesting perspective for understanding the pathophysiology of the disease. The nature of the unidentified targets and the pathogenicity of ANCAs, however, remain to be assessed.

Adolescent↗
PubMed2003

Anetoderma and its prothrombotic abnormalities.

BACKGROUND: Anetoderma is characterized by circumscribed areas of flaccid skin due to the loss of elastic tissue in the dermis. It may be primary or secondary to various dermatoses. The primary form has been reported in association with autoimmune diseases and recently with antiphospholipid antibodies. Its etiology remains unknown. OBJECTIVES: To analyze clinical and laboratory data from a series of patients with anetoderma referred in our university reference center for connective tissue disorders. PATIENTS AND METHODS: All the consecutive patients with histologically confirmed anetoderma followed in our clinic from 1996 to 2001 were enrolled in this study. Laboratory investigations included the screening for prothrombotic abnormalities and classical immunological investigations for systemic lupus erythematosus. Clinical and laboratory data were analyzed retrospectively. RESULTS: Anetoderma was primary in 9 cases and secondary to lupus profundus in 2 cases. Prothrombotic abnormalities were detected in 10 patients (9/9 with primary and 1/2 with secondary anetoderma). Antiphospholipid antibodies were detected in 9 patients. Only 4 patients fulfilled criteria for definite antiphospholipid syndrome which was primary for 3 and associated with systemic lupus erythematosus in the other. CONCLUSION: Patients with anetoderma should be evaluated for the possible presence of a prothrombotic state and warned of its potential risks when present.

Adolescent↗
PubMed2003

[Classification of dermatologic manifestations in lupus erythematosus].

Various dermatologic manifestations are observed in the different lupus subsets. Lupus lesions are characterized by a dermo-epidermal dermatitis. Other lesions, vascular or non vascular, are essentially present in association with systemic lupus erythematosus. Acute, subacute and chronic lupus erythematosus are distinguishable according to their clinical aspects, pathological features and evolution. Acute lesions are either localized to the midface or widespread. Subacute lesions may be annular or psoriasiform. Chronic lupus erythematosus includes localized or widespread discoid lupus, lupus tumidus, chilblain lupus and panniculitis. Therapy of cutaneous lupus is mainly based on antimalarials and avoidance of sun irradiation. In refractory cutaneous lupus, no universal guidelines are currently available. Except for acrosyndromes and urticaria-like lesions, vascular lesions may be due to vasculitis or thrombosis. An accurate diagnosis is absolutely necessary since therapy is different in thrombosis and vasculitis. Non vascular and non lupus lesions are numerous, some of them require special treatment such as dapsone for bullous lupus.

Acitretin↗
PubMed2002

[How to recognize scleroderma].

Systemic sclerosis is a multisystem disorder characterized by the presence of connective tissue sclerosis, vascular abnormalities concerning small-sized arteries and the microcirculation, and autoimmune changes. This disease is usually preceded by Raynaud's phenomenon. Cutaneous changes are useful for diagnosis. Sclerosis is initially proeminent on hands with frequently pitted scars. Telangiectases and calcium deposits are mainly observed in a good prognosis subset of the disease (CREST) characterized by the association of limited skin involvement and anti-centromere antibodies with possible late onset of pulmonary hypertension. The bad prognosis subset is associated with diffuse cutaneous sclerosis, pulmonary fibrosis, gastrointestinal disease, myocardial involvement and anti-topo-isomerase I antibodies. Histological skin changes are similar to those observed in morphea. In morphea, there is only localized sclerosis of the skin with highly variable aesthetic disability. The condition may be subdivided clinically into the following types: circumscribed plaques, linear morphea and frontoparietal lesions. The last two forms may be associated with atrophy of the underlying anatomic structures. Many other diseases may have a scleroderma-like appearance. In these diseases, nail folds abnormalities, visceral involvement and immunological changes are usually different from those observed in systemic scleroderma.

Diagnosis, Differential↗
PubMed2002

Prognostic value of quantitative Kaposi sarcoma--associated herpesvirus load in posttransplantation Kaposi sarcoma.

Organ transplant recipients have a higher risk of Kaposi sarcoma (KS). A quantitative real-time polymerase chain reaction assay was developed to evaluate KS-associated herpesvirus (KSHV) as a prognostic tool in transplant recipients with KS. Forty-three patients who developed KS after transplantation were included in a cross-sectional study to correlate virus load with transplantation or KS parameters. Seventeen patients (40%) had KSHV viremia (>100 copies/microg of DNA; median, 6067 copies/microg of DNA). Factors associated with these levels of viremia by univariate analysis were progression of KS (P=.00002), time from KS diagnosis (P=.0007), actual stage of KS (P=.006), initial stage of KS (P=.22), graft loss (P=.013), and time from transplantation (P=.0246). Disease progression remained associated with KSHV viremia in a multivariate analysis (P=.01). Thus, quantification of KSHV load in peripheral blood mononuclear cells could represent a useful tool for monitoring transplant recipients with KS.

Cohort Studies↗
PubMed2002

[Prevention of skin complications from immunodepression in organ transplantation].

As skin diseases after organ transplantation are often disabling and sometimes lethal, it is essential to develop preventive measures. The group "Skin and organ transplantation" of the French Society of Dermatology concentrated one's efforts on skin carcinomas and Kaposis's sarcoma. Skin carcinomas are the most frequent tumors observed in transplant recipients. Many factors participate to their pathogenesis such as genetic, environmental and iatrogenic. Their prevalence is very high in elderly transplant recipients with fair complexion, long time of immunosuppression, long time of sun exposure, multiple keratoses and warts. Preventive measures consist educational training, sun avoidance and treatment of keratoses. Transplant recipients who develop Kaposi's disease are all infected with Human Herpes Virus 8 (HHV-8). HHV-8 promoinfection occurred frequently before transplantation. Cofactors involved in the development of Kaposi' sarcoma will be probably identified by the results of an ongoing national prospective study of kidney transplant recipients. Immunosuppressive therapy should be tapered to the lowest possible level compatible with allograft function.

Humans↗